The Polycythemias

Published on 04/03/2015 by admin

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Chapter 28 The Polycythemias

Marina Kremyanskaya, Vesna Najfeld, John Mascarenhas, Ronald Hoffman

Table 28-1 Differential Diagnosis of the Polycythemias

Relative or Spurious Polycythemia
1. Decreased plasma volume—reduced fluid intake, marked loss of body fluids (diaphoresis, vomiting, diarrhea, “third spacing”) 2. Gaisböck syndrome 3. Overfilling of blood in collection vacuum tubes
Absolute Polycythemia
1. Secondary polycythemia A. Acquired Hypoxia • Pulmonary disease • Cyanotic congenital heart disease • Hypoventilation syndromes: sleep apnea, Pickwickian syndrome • High altitude • Smokers’ polycythemia, hookah polycythemia, carbon monoxide intoxication caused by industrial exposure Postrenal transplantation erythrocytosis Aberrant erythropoietin production • Tumors: renal cell carcinoma, Wilms tumor, hepatic carcinoma, uterine leiomyomata, virilizing ovarian tumors, vascular cerebellar tumors • Miscellaneous renal and hepatic disorders: solitary renal cysts, polycystic kidney disease, renal artery stenosis hydronephrosis, viral hepatitis Endocrine disorders: Cushing syndrome, primary aldosteronism Androgen use Erythropoietin use B. Congenital Polycythemias • Abnormal high-affinity hemoglobin variants • Bisphosphoglycerate deficiency • Congenital methemoglobinemia • Chuvash polycythemia (von Hippel Lindau mutations) • Prolyl hydroxylase mutations • Hypoxia-inducible factor gene mutations 2. Primary polycythemias • Primary congenital and familial polycythemia • Polycythemia vera

Figure 28-1 EVOLUTION OF POLYCYTHEMIA VERA (PV).

MF, Myelofibrosis.

Table 28-2 International Working Group for Myelofibrosis Research and Recommended Treatment Criteria for Post-PV MF

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Required Criteria:
1. Documentation of a previous diagnosis of polycythemia vera as defined by the WHO criteria 2. Bone marrow fibrosis grade 2-3 (on 0-3 scale) or grade 3-4 (on 0-4 scale)
Additional Criteria (Two Are Required):

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